A new prediction model for ventricular arrhythmias in arrhythmogenic right ventricular cardiomyopathy

Research output: Contribution to journalArticle


AIMS: Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVC) is characterized by ventricular arrhythmias (VAs) and sudden cardiac death (SCD). We aimed to develop a model for individualized prediction of incident VA/SCD in ARVC patients. METHODS AND RESULTS: Five hundred and twenty-eight patients with a definite diagnosis and no history of sustained VAs/SCD at baseline, aged 38.2 ± 15.5 years, 44.7% male, were enrolled from five registries in North America and Europe. Over 4.83 (interquartile range 2.44-9.33) years of follow-up, 146 (27.7%) experienced sustained VA, defined as SCD, aborted SCD, sustained ventricular tachycardia, or appropriate implantable cardioverter-defibrillator (ICD) therapy. A prediction model estimating annual VA risk was developed using Cox regression with internal validation. Eight potential predictors were pre-specified: age, sex, cardiac syncope in the prior 6 months, non-sustained ventricular tachycardia, number of premature ventricular complexes in 24 h, number of leads with T-wave inversion, and right and left ventricular ejection fractions (LVEFs). All except LVEF were retained in the final model. The model accurately distinguished patients with and without events, with an optimism-corrected C-index of 0.77 [95% confidence interval (CI) 0.73-0.81] and minimal over-optimism [calibration slope of 0.93 (95% CI 0.92-0.95)]. By decision curve analysis, the clinical benefit of the model was superior to a current consensus-based ICD placement algorithm with a 20.6% reduction of ICD placements with the same proportion of protected patients (P < 0.001). CONCLUSION: Using the largest cohort of patients with ARVC and no prior VA, a prediction model using readily available clinical parameters was devised to estimate VA risk and guide decisions regarding primary prevention ICDs (www.arvcrisk.com).


  • Julia Cadrin-Tourigny
  • Laurens P. Bosman
  • Anna Nozza
  • Weijia Wang
  • Rafik Tadros
  • Aditya Bhonsale
  • Mimount Bourfiss
  • Annik Fortier
  • Øyvind H. Lie
  • Ardan M. Saguner
  • Anneli Svensson
  • Antoine Andorin
  • Crystal Tichnell
  • Brittney Murray
  • Katja Zeppenfeld
  • Maarten P. van den Berg
  • Folkert W. Asselbergs
  • Arthur A.M. Wilde
  • Andrew D. Krahn
  • Mario Talajic
  • Lena Rivard
  • Stephen Chelko
  • Stefan L. Zimmerman
  • Ihab R. Kamel
  • Jane E. Crosson
  • Daniel P. Judge
  • Sing Chien Yap
  • Jeroen F. van der Heijden
  • Harikrishna Tandri
  • Jan D.H. Jongbloed
  • Marie Claude Guertin
  • J. Peter van Tintelen
  • Firat Duru
  • Kristina H. Haugaa
  • Paul Khairy
  • Richard N.W. Hauer
  • Hugh Calkins
  • Anneline S.J.M. Te Riele
  • Cynthia A. James
External organisations
  • Johns Hopkins Hospital
  • University Medical Center Utrecht
  • Oslo university hospital
  • University of Oslo
  • Linköping University Hospital
  • Leiden University Medical Centre
  • University Medical Center Groningen
  • University College London
  • University of British Columbia
  • Erasmus University Medical Center
  • Interuniversity Cardiology Institute of the Netherlands
  • University of Montreal, Canada
  • University Hospital of Zurich
  • University of Amsterdam
  • Montreal Heart Institute
  • Academic Medical Center of University of Amsterdam (AMC)
Research areas and keywords

Subject classification (UKÄ) – MANDATORY

  • Cardiac and Cardiovascular Systems


  • Arrhythmogenic right ventricular cardiomyopathy, Implantable cardioverter-defibrillators, Sudden cardiac death, Ventricular arrhythmias
Original languageEnglish
Pages (from-to)1850-1858
Number of pages9
JournalEuropean Heart Journal
Issue number23
Publication statusPublished - 2019 Jun
Publication categoryResearch